Popliteal Ptergyium Syndrome
Popliteal Ptergyium Syndrome (PPS) is a very rare genetic condition of unknown etiology. It is characterized by webbing of the joints, primary the knees, ankles, and elbows. Abnormalities in PPS include:
Musculoskeletal abnormalities are manifested by formation of multiple ptergyia (webbing) across the flexor aspect of the joints resulting in growth retardation, syndactyly, and toenail dysplasia. PPS is quite rare, occurring in 1 in 300,000 live births. Individuals with PPS have normal intelligance. The principal hypothesis for the cause of PPS is an arrest of development of parts based on an imbalance between mesodermal migration and ectodermal proliferation.
Common complaints in individuals with PPS include gait disturbances, cosmesis, difficulty in performing daily activities, quadriceps pain, and quick fatiguing. Mobility of the individual is affected due to crouch gait, which varies depending on the severity of contracture and arc of motion in the nonfunctional range. Many patients are wheelchair bound for locomotion, if it affects both lower limbs with severe magnitude.
Constant presence of ptergyia in PPS requires orthopedic management. At the Paley Institute, our approach to managing this condition is by extensive soft tissue release, application of an external fixation device followed by casting and prolonged bracing. Our aim is to make the patient independent and ambulatory, and also to minimize the recurrence and thereby minimizing revision surgeries without causing damage to the neurological and joint structures.
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